Abstract
Mast cells arise from pluripotent stem cells. From the original identification of mast cells in the late 1800s, our understanding of these cells’ normal function and role in pathologic disease has expanded greatly; and the understanding of mastocytosis has led to advances in classification and treatment of these diseases. The term mastocytosis describes a group of disorders characterized by abnormal proliferation of mast cells. Mast cell numbers are increased and pathologically infiltrate various organ systems, resulting in a spectrum of disorders from cutaneous mastocytosis (more common in children) to multiple subvariants of systemic mastocytosis, mast cell leukemia, and mastocytomas. Diagnostic criteria have been modified recently to aid in classifying the type of disease, which allows for better determination of both the prognosis and treatment. While treatment is largely symptomatic, with important focus on the management of anaphylaxis, several promising therapeutic targets and agents have recently been identified which may lead to improved survival in more advanced subtypes of disease. These therapies include several tyrosine kinase inhibitors which combat the activating KIT mutations present in most patients with aggressive mastocytosis.
Similar content being viewed by others
References
Abbas AK, Lichtman AHH, Pillai S. Cellular and molecular immunology E-Book. Philadelphia: Elsevier Health Sciences; 2017.
Akin C. Mast cell activation syndromes. J Allergy Clin Immunol. 2017;140(2):349–55.
Akin C, Valent P. Diagnostic criteria and classification of mastocytosis in 2014. Immunol Allergy Clin N Am. 2014;34(2):207–18.
Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: proposed diagnostic criteria. J Allergy Clin Immunol. 2010;126(6):1099–1104.e4.
Alandijani S, Casale TB, Ledford DK, Lockey RF. 3-generational familial tryptasemia with multiple clinical presentations. J Allergy Clin Immunol. 2017;139(2):AB166.
Alvarez-Twose I, et al. Imatinib in systemic mastocytosis: a phase IV clinical trial in patients lacking exon 17 KIT mutations and review of the literature. Oncotarget. 2016;8(40):68950–63.
Arber DA, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–405.
Arock M, et al. KIT mutation analysis in mast cell neoplasms: recommendations of the European Competence Network on Mastocytosis. Leukemia. 2015;29(6):1223–32.
Ashinoff R, Soter NA, Freedberg IM. Solitary mastocytoma in an adult. J Dermatol Surg Oncol. 1993;19(5):487–8.
Barete S, et al. Systemic mastocytosis and bone involvement in a cohort of 75 patients. Ann Rheum Dis. 2010;69(10):1838–41.
Barete S, et al. Long-term efficacy and safety of cladribine (2-CdA) in adult patients with mastocytosis. Blood. 2015;126(8):1009–16; quiz 1050.
Beaven M. Our perception of the mast cell from Paul Ehrlich to now. Eur. J. Immunol. 2009;39(1):11–25.
Bell MC, Jackson DJ. Prevention of anaphylaxis related to mast cell activation syndrome with omalizumab. Ann Allergy Asthma Immunol. 2012;108(5):383–4.
Biosse-Duplan M, Baroukh B, Dy M, de Vernejoul M-C, Saffar J-L. Histamine promotes osteoclastogenesis through the differential expression of histamine receptors on osteoclasts and osteoblasts. Am J Pathol. 2009;174(4):1426–34.
Bonadonna P, et al. Venom immunotherapy in patients with clonal mast cell disorders: efficacy, safety, and practical considerations. J Allergy Clin Immunol Pract. 2013;1(5):474–8.
Borate U, Mehta A, Reddy V, Tsai M, Josephson N, Schnadig I. Treatment of CD30-positive systemic mastocytosis with brentuximab vedotin. Leuk Res. 2016;44:25–31.
Brazzelli V, et al. Narrow-band UVB phototherapy and psoralen-ultraviolet A photochemotherapy in the treatment of cutaneous mastocytosis: a study in 20 patients. Photodermatol Photoimmunol Photomed. 2016;32(5–6):238–46.
Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin N Am. 2014;34(2):283–95.
Brockow K, Metcalfe DD. Mastocytosis. Chemical Immunology and Allergy. 2010;95:110–24.
Brockow K, et al. Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease. Arch Dermatol. 2002;138(6):785–90.
Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008;63(2):226–32.
Broesby-Olsen S, et al. Omalizumab prevents anaphylaxis and improves symptoms in systemic mastocytosis: efficacy and safety observations. Allergy. 2017;73(1):230–8.
Butterfield JH. Response of severe systemic mastocytosis to interferon alpha. Br J Dermatol. 1998;138(3):489–95.
Butterfield JH, Kao PC, Klee GG, Yocum MW. Aspirin idiosyncrasy in systemic mast cell disease: a new look at mediator release during aspirin desensitization. Mayo Clin Proc. 1995;70(5):481–7.
Butterfield JH, Tefferi A, Kozuh GF. Successful treatment of systemic mastocytosis with high-dose interferon-alfa: long-term follow-up of a case. Leuk Res. 2005;29(2):131–4.
Carter MC, Robyn JA, Bressler PB, Walker JC, Shapiro GG, Metcalfe DD. Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis. J Allergy Clin Immunol. 2007;119(6):1550–1.
Carter MC, Metcalfe DD, Komarow HD. Mastocytosis. Immunol Allergy Clin N Am. 2014;34(1):181–96.
Castells MC, Irani AM, Schwartz LB. Evaluation of human peripheral blood leukocytes for mast cell tryptase. J Immunol. 1987;138(7):2184–9.
Chandesris M-O, et al. Midostaurin in advanced systemic mastocytosis. N Engl J Med. 2016;374(26):2605–7.
Cohen SS, et al. Epidemiology of systemic mastocytosis in Denmark. Br J Haematol. 2014;166(4):521–8.
Correia O, Duarte AF, Quirino P, Azevedo R, Delgado L. Cutaneous mastocytosis: two pediatric cases treated with topical pimecrolimus. Dermatol Online J. 2010;16(5):8.
Cruse G, Metcalfe DD, Olivera A. Functional deregulation of KIT: link to mast cell proliferative diseases and other neoplasms. Immunol Allergy Clin N Am. 2014;34(2):219–37.
de Melo Campos P, et al. Familial systemic mastocytosis with germline KIT K509I mutation is sensitive to treatment with imatinib, dasatinib and PKC412. Leuk Res. 2014;38(10):1245–51.
DeAngelo DJ, et al. Efficacy and safety of midostaurin in patients with advanced systemic mastocytosis: 10-year median follow-up of a phase II trial. Leukemia. 2018;32(2):470–8.
Delaporte E, et al. Interferon-α in combination with corticosteroids improves systemic mast cell disease. Br J Dermatol. 1995;132(3):479–82.
Dobigny C, Saffar J-L. H1 and H2 histamine receptors modulate osteoclastic resorption by different pathways: evidence obtained by using receptor antagonists in a rat synchronized resorption model. J Cell Physiol. 1997;173(1):10–8.
Doyle LA, Hornick JL. Pathology of extramedullary mastocytosis. Immunol Allergy Clin N Am. 2014;34(2):323–39.
Ellis JM. Urticaria pigmentosa; a report of a case with autopsy. Arch Pathol. 1949;48(5):426–35.
Escribano L, et al. Immunophenotypic characterization of human bone marrow mast cells. A flow cytometric study of normal and pathological bone marrow samples. Anal Cell Pathol. 1998;16(3):151–9.
Escribano L, Akin C, Castells M, Schwartz LB. Current options in the treatment of mast cell mediator-related symptoms in mastocytosis. Inflamm Allergy Drug Targets. 2006;5(1):61–77.
Fabbro D, et al. PKC412 – a protein kinase inhibitor with a broad therapeutic potential. Anticancer Drug Des. 2000;15(1):17–28.
Franklin Adkinson N Jr, et al. Middleton’s allergy E-book: principles and practice. London: Elsevier Health Sciences; 2013.
Garriga MM, Friedman MM, Metcalfe DD. A survey of the number and distribution of mast cells in the skin of patients with mast cell disorders. J Allergy Clin Immunol. 1988;82(3 Pt 1):425–32.
Godt O, Proksch E, Streit V, Christophers E. Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. Dermatology. 1997;195(1):35–9.
González de Olano D, et al. Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis. J Allergy Clin Immunol. 2008;121(2):519–26.
Gotlib J. Tyrosine kinase inhibitors in the treatment of eosinophilic neoplasms and systemic mastocytosis. Hematol Oncol Clin North Am. 2017;31(4):643–61.
Gotlib J, et al. Efficacy and safety of midostaurin in advanced systemic mastocytosis. N Engl J Med. 2016;374(26):2530–41.
Growney JD, et al. Activation mutations of human c-KIT resistant to imatinib mesylate are sensitive to the tyrosine kinase inhibitor PKC412. Blood. 2005;106(2):721–4.
Gülen T, Hägglund H, Dahlén B, Nilsson G. Mastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic disease. J Intern Med. 2016;279(3):211–28.
Hartmann K, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 2016;137(1):35–45.
Hauswirth AW, et al. Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: report of five cases and review of the literature. Leuk Res. 2004;28(3):249–57.
Heide R, et al. Mastocytosis in children: a protocol for management. Pediatr Dermatol. 2008;25(4):493–500.
Hermans MAW, et al. Management around invasive procedures in mastocytosis: an update. Ann Allergy Asthma Immunol. 2017;119(4):304–9.
Hermine O, et al. Case-control cohort study of patients’ perceptions of disability in mastocytosis. PLoS One. 2008;3(5):e2266.
Hochhaus A, et al. Nilotinib in patients with systemic mastocytosis: analysis of the phase 2, open-label, single-arm nilotinib registration study. J Cancer Res Clin Oncol. 2015;141(11):2047–60.
Horan RF, Sheffer AL, Austen KF. Cromolyn sodium in the management of systemic mastocytosis. J Allergy Clin Immunol. 1990;85(5):852–5.
Horny HP, et al. Diagnostic value of immunostaining for tryptase in patients with mastocytosis. Am J Surg Pathol. 1998;22(9):1132–40.
Horny H-P, Sotlar K, Valent P. Mastocytosis: immunophenotypical features of the transformed mast cells are unique among hematopoietic cells. Immunol Allergy Clin N Am. 2014;34(2):315–21.
International Agency for Research on Cancer and World Health Organization. WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization; Lyon, France. 2008.
Jawhar M, et al. Molecular profiling of myeloid progenitor cells in multi-mutated advanced systemic mastocytosis identifies KIT D816V as a distinct and late event. Leukemia. 2015;29(5):1115–22.
Klaiber N, Kumar S, Irani A-M. Mastocytosis in children. Curr Allergy Asthma Rep. 2017;17(11):80.
Kluin-Nelemans HC, et al. Response to interferon alfa-2b in a patient with systemic mastocytosis. N Engl J Med. 1992;326(9):619–23.
Kovalszki A, Weller PF. Eosinophilia in mast cell disease. Immunol Allergy Clin N Am. 2014;34(2):357–64.
Krauth M-T, Mirkina I, Herrmann H, Baumgartner C, Kneidinger M, Valent P. Midostaurin (PKC412) inhibits immunoglobulin E-dependent activation and mediator release in human blood basophils and mast cells. Clin Exp Allergy. 2009;39(11):1711–20.
Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W. Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature. J Eur Acad Dermatol Venereol. 2012;26(12):1565–71.
Laroche M, Livideanu C, Paul C, Cantagrel A. Interferon alpha and pamidronate in osteoporosis with fracture secondary to mastocytosis. Am J Med. 2011;124(8):776–8.
Lehmann T, et al. Severe osteoporosis due to systemic mast cell disease: successful treatment with interferon alpha-2B. Br J Rheumatol. 1996;35(9):898–900.
Lehner E. II. Beiträge zur Klinik und Histologie der Urticaria pigmentosa. Dermatology. 1926;46(2):87–93.
Lieberoth S, Thomsen SF. Cutaneous and gastrointestinal symptoms in two patients with systemic mastocytosis successfully treated with omalizumab. Case Rep Med. 2015;2015:903541.
Lim JL, Stern RS. High levels of ultraviolet B exposure increase the risk of non-melanoma skin cancer in psoralen and ultraviolet A-treated patients. J Invest Dermatol. 2005;124(3):505–13.
Lim AYN, Ostor AJK, Love S, Crisp AJ. Systemic mastocytosis: a rare cause of osteoporosis and its response to bisphosphonate treatment. Ann Rheum Dis. 2005;64(6):965–6.
Lim KH, Pardanani A, Butterfield JH, Li C-Y, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009;84(12):790–4.
Lock AD, McNamara CJ, Rustin MHA. Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine. Clin Exp Dermatol. 2014;40(2):142–5.
Lortholary O, et al. Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study. Lancet. 2017;389(10069):612–20.
Lyons JJ, et al. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nat Genet. 2016;48(12):1564–9.
Maeda H, et al. Requirement of c-kit for development of intestinal pacemaker system. Development. 1992;116(2):369–75.
Markey AC, Churchill LJ, MacDonald DM. Human cutaneous mast cells – a study of fixative and staining reactions in normal skin. Br J Dermatol. 1989;120(5):625–31.
Matito A, et al. Management of anesthesia in adult and pediatric mastocytosis: a study of the Spanish Network on Mastocytosis (REMA) based on 726 anesthetic procedures. Int Arch Allergy Immunol. 2015;167(1):47–56.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I. Cutaneous mastocytosis in adults and children: new classification and prognostic factors. Immunol Allergy Clin N Am. 2018;38(3):351–63.
Méni C, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol. 2015;172(3):642–51.
Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. 1991a;96(3):2S–4S.
Metcalfe DD. The liver, spleen, and lymph nodes in mastocytosis. J Invest Dermatol. 1991b;96(3):45S–6S.
Monnier J, et al. Mast cell sarcoma: new cases and literature review. Oncotarget. 2016;7(40):66299–309.
Morgado JMT, et al. Immunophenotyping in systemic mastocytosis diagnosis: ‘CD25 positive’ alone is more informative than the ‘CD25 and/or CD2’ WHO criterion. Mod Pathol. 2012;25(4):516–21.
Moura DS, Georgin-Lavialle S, Gaillard R, Hermine O. Neuropsychological features of adult mastocytosis. Immunol Allergy Clin N Am. 2014;34(2):407–22.
Naumann N, et al. Incidence and prognostic impact of cytogenetic aberrations in patients with systemic mastocytosis. Genes Chromosom Cancer. 2018;57:252–9.
Nettleship E. RARE CASES OF IRITIS IN CHILDREN NEAR THE AGE OF PUBERTY. WITH REMARKS. Lancet. 1876;107(2733):86–7.
Nicoloro-SantaBarbara J, Lobel M, Wolfe D. Psychosocial impact of mast cell disorders: pilot investigation of a rare and understudied disease. J Health Psychol. 2017;22(10):1277–88.
Onnes MC, Tanno LK, Elberink JNGO. Mast cell clonal disorders: classification, diagnosis and management. Curr Treat Options Allergy. 2016;3(4):453–64.
Pardanani A. Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management. Am J Hematol. 2016;91(11):1146–59.
Pardanani A, et al. Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies. Blood. 2009;114(18):3769–72.
Pardanani A, Finke C, Abdelrahman RA, Lasho TL, Hanson CA, Tefferi A. Increased circulating IL-2Rα (CD25) predicts poor outcome in both indolent and aggressive forms of mastocytosis: a comprehensive cytokine–phenotype study. Leukemia. 2013;27(6):1430–3.
Parker RI. Hematologic aspects of mastocytosis: I: bone marrow pathology in adult and pediatric systemic mast cell disease. J Invest Dermatol. 1991;96(3):47S–51S.
Parker RI. Hematologic aspects of systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14(3):557–68.
Rose S. Rapid Responses to Avapritinib (BLU-285) in Mastocytosis. Cancer Discov. 2018;8(2):133.
Ribatti D. The development of human mast cells. An historical reappraisal. Exp Cell Res. 2016;342(2):210–5.
Rogers MP, Bloomingdale K, Murawski BJ, Soter NA, Reich P, Austen KF. Mixed organic brain syndrome as a manifestation of systemic mastocytosis. Psychosom Med. 1986;48(6):437–47.
Rossini M, et al. Bone mineral density, bone turnover markers and fractures in patients with indolent systemic mastocytosis. Bone. 2011;49(4):880–5.
Rossini M, et al. Bone involvement and osteoporosis in mastocytosis. Immunol Allergy Clin N Am. 2014;34(2):383–96.
Rossini M, et al. Prevalence, pathogenesis, and treatment options for mastocytosis-related osteoporosis. Osteoporos Int. 2016;27(8):2411–21.
Rothe MJ, Grant-Kels JM, Makkar HS. Mast cell disorders: kids are not just little people. Clin Dermatol. 2016;34(6):760–6.
Ruëff F, Placzek M, Przybilla B. Mastocytosis and Hymenoptera venom allergy. Curr Opin Allergy Clin Immunol. 2006;6(4):284–8.
Sadashiv S, Bower K, Bower K, Sahovic E, Bunker M, Christou A. Use of octreotide for relief of gastro-intestinal (GI) symptoms in systemic mastocytosis. Hematol Oncol Stem Cell Ther. 2013;6(2):72–5.
Schuch A, Brockow K. Mastocytosis and anaphylaxis. Immunol Allergy Clin N Am. 2017;37(1):153–64.
Schwaab J, et al. Comprehensive mutational profiling in advanced systemic mastocytosis. Blood. 2013;122(14):2460–6.
Shih AR, Deshpande V, Ferry JA, Zukerberg L. Clinicopathological characteristics of systemic mastocytosis in the intestine. Histopathology. 2016;69(6):1021–7.
Siebenhaar F, Kühn W, Zuberbier T, Maurer M. Successful treatment of cutaneous mastocytosis and Ménière disease with anti-IgE therapy. J Allergy Clin Immunol. 2007;120(1):213–5.
Siebenhaar F, et al. Rupatadine improves quality of life in mastocytosis: a randomized, double-blind, placebo-controlled trial. Allergy. 2013;68(7):949–52.
Siebenhaar F, Akin C, Bindslev-Jensen C, Maurer M, Broesby-Olsen S. Treatment strategies in mastocytosis. Immunol Allergy Clin N Am. 2014;34(2):433–47.
Simon J, et al. Interest of interferon alpha in systemic mastocytosis. The French experience and review of the literature. Pathol Biol. 2004;52(5):294–9.
Simons FER, et al. World Allergy Organization Anaphylaxis Guidelines: 2013 update of the evidence base. Int Arch Allergy Immunol. 2013;162(3):193–204.
Simons FER, et al. 2015 update of the evidence base: World Allergy Organization anaphylaxis guidelines. World Allergy Organ J. 2015;8(1):32.
Sokol H, et al. Gastrointestinal involvement and manifestations in systemic mastocytosis. Inflamm Bowel Dis. 2010;16(7):1247–53.
Sokol H, et al. Gastrointestinal manifestations in mastocytosis: a study of 83 patients. J Allergy Clin Immunol. 2013;132(4):866–73.e1–3.
Sokol KC, Ghazi A, Kelly BC, Grant JA. Omalizumab as a desensitizing agent and treatment in mastocytosis: a review of the literature and case report. J Allergy Clin Immunol Pract. 2014;2(3):266–70.
Soter NA, Frank Austen K, Wasserman SI. Oral disodium cromoglycate in the treatment of systemic mastocytosis. N Engl J Med. 1979;301(9):465–9.
Sotlar K, et al. CD25 indicates the neoplastic phenotype of mast cells. Am J Surg Pathol. 2004;28(10):1319–25.
Sotlar K, et al. Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis. Mod Pathol. 2011;24(4):585–95.
Sperr WR, et al. Elevated tryptase levels selectively cluster in myeloid neoplasms: a novel diagnostic approach and screen marker in clinical haematology. Eur J Clin Investig. 2009;39(10):914–23.
Sukrithan VK, Salamon JN, Berulava G, Sibinga NE, Verma A. Systemic mastocytosis presenting as cardiac tamponade with CD25(+) pericardial mast cells. Clin Case Rep. 2016;4(3):279–81.
Theoharides TC, Valent P, Akin C. Mast cells, mastocytosis, and related disorders. N Engl J Med. 2015;373(2):163–72.
Thompson JH. A CASE OF FACTITIOUS URTICARIA. Lancet. 1893;141(3634):924.
Travis WD, Li CY, Bergstralh EJ, Yam LT, Swee RG. Systemic mast cell disease. Analysis of 58 cases and literature review. Medicine. 1988;67(6):345–68.
Turner PJ, Kemp AS, Rogers M, Mehr S. Refractory symptoms successfully treated with leukotriene inhibition in a child with systemic mastocytosis. Pediatr Dermatol. 2011;29(2):222–3.
Ustun C, et al. Hematopoietic stem-cell transplantation for advanced systemic mastocytosis. J Clin Oncol. 2014;32(29):3264–74.
Uzzaman A, Maric I, Noel P, Kettelhut BV, Metcalfe DD, Carter MC. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer. 2009;53(4):629–34.
Valent P. Diagnosis and management of mastocytosis: an emerging challenge in applied hematology. Hematology Am Soc Hematol Educ Program. 2015;2015:98–105.
Valent P, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603–25.
Valent P, Sperr WR, Schwartz LB, Horny H-P. Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol. 2004;114(1):3–11; quiz 12.
Valent P, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Investig. 2007;37(6):435–53.
Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017a;129(11):1420–7.
Valent P, et al. Advances in the classification and treatment of mastocytosis: current status and outlook toward the future. Cancer Res. 2017b;77(6):1261–70.
Valent P, et al. Midostaurin: a magic bullet that blocks mast cell expansion and activation. Ann Oncol. 2017c;28(10):2367–76.
van den Elzen MT, et al. Effectiveness and safety of antihistamines up to fourfold or higher in treatment of chronic spontaneous urticaria. Clin Transl Allergy. 2017;7(1):4.
van Doormaal JJ, et al. Prevalence of indolent systemic mastocytosis in a Dutch region. J Allergy Clin Immunol. 2013;131(5):1429–1431.e1.
Vega-Ruiz A, et al. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. 2009;33(11):1481–4.
Veien M, Szlam F, Holden JT, Yamaguchi K, Denson DD, Levy JH. Mechanisms of nonimmunological histamine and tryptase release from human cutaneous mast cells. Anesthesiology. 2000;92(4):1074–81.
Vieira dos Santos R, et al. Topical sodium cromoglicate relieves allergen- and histamine-induced dermal pruritus. Br J Dermatol. 2010;162(3):674–6.
Weiler CR, Butterfield J. Mast cell sarcoma: clinical management. Immunol Allergy Clin N Am. 2014;34(2):423–32.
Wiechers T, et al. Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis. J Allergy Clin Immunol. 2015;136(6):1581–1590.e3.
Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res. 2001;25(7):519–28.
Zaheer S, LeBoff M, Lewiecki EM. Denosumab for the treatment of osteoporosis. Expert Opin Drug Metab Toxicol. 2015;11(3):461–70.
Zhang LY, et al. A novel K509I mutation of KIT identified in familial mastocytosis-in vitro and in vivo responsiveness to imatinib therapy. Leuk Res. 2006;30(4):373–8.
Zuberbier T, et al. The EAACI/GA2LEN/EDF/WAO guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update. Allergy. 2014;69(7):868–87.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Section Editor information
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this entry
Cite this entry
Alagheband, S., Cranford, C., Stewart, P. (2019). Mast Cell Disorders and Anaphylaxis. In: Craig, T., Ledford, D. (eds) Allergy and Asthma. Springer, Cham. https://doi.org/10.1007/978-3-319-58726-4_30-1
Download citation
DOI: https://doi.org/10.1007/978-3-319-58726-4_30-1
Received:
Accepted:
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-58726-4
Online ISBN: 978-3-319-58726-4
eBook Packages: Springer Reference MedicineReference Module Medicine